1/21. pregnancy with primary tubal placental site trophoblastic tumor--A case report and literature review.Placental site trophoblastic tumor (PSTT) is a rare trophoblastic neoplasm with malignant potential. The diagnosis and prognosis of this rare disease remain difficult. A case of tubal PSTT with the primary manifestation of internal bleeding at 30 weeks' gestation is presented. Emergency exploratory laparotomy and right partial salpingectomy were performed initially. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were done immediately after cesarean section at 34 weeks' gestation. No further adjuvant therapy was given after surgery. No evidence of tumor recurrence or signs of metastasis were noted during 12 months of follow-up. Heterotopic pregnancy, one with intrauterine normal pregnancy and the other with implanting in the right fallopian tube and placental site trophoblastic tumor transformation, was proposed. To our knowledge, this is the first such case in the English literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/21. Clinicopathologic and cytogenetic analyses of three cases of primary uterine non-Hodgkin's lymphoma.Primary uterine non-Hodgkin's lymphoma (NHL) is an extremely rare disease. To accumulate more information on clinical data, we report three cases of primary uterine NHL with apparently the first demonstration of karyotypic analysis. Histological diagnosis was diffuse large B cell type in all patients. Two of them with advanced stage showed chemoresistance and a short survival. The remaining case with early stage showed an uneventful course following operation. No common chromosomal abnormality was detected. The therapeutic strategy for uterine NHL might therefore be similar to that for other types of aggressive NHL, although a larger study is needed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/21. Primary uterine lymphoma: a case report.Primary lymphoma of the uterus is a rare disease, the reported characteristic MR imaging findings being homogeneous intermediate signal intensity of the indistinct mass on T1- and T2-weighted images, and the preservation of endometrial lining and uterine architecture. We report a case of primary uterine lymphoma which showed tumoral necrosis, endometrial disruption and diffuse anterior vaginal wall involvement.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/21. Adrenal metastatic placental site trophoblastic tumor. Case report.Placental site trophoblastic tumor is a rare disease. Since its first description in the literature only about 100 cases have been reported. Initially considered a benign pathology, its malignant potential with metastases was later recognized. About 30% of the cases described metastatised, thereby aggravating the prognosis. The therapy is surgical and in cases of metastatised disease has to be supplemented by chemotherapy. A case of placental site trophoblastic tumour with a single metastasis to the left adrenal gland treated with adrenalectomy and chemotherapy is reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/21. Human pulmonary dirofilariasis in a patient whose clinical condition altered during follow-up.Pulmonary dirofilariasis is a rare disease. We have experienced a case which developed pleural effusion while being followed, rendering it difficult to establish the diagnosis. The patient, a 53-year-old woman, had previously undergone two operations; one for uterine cancer and another for breast cancer. She developed a cough in February 2002, and chest computed tomography (CT) scans disclosed a nodular mass in the right lung. A biopsy revealed a fibrous nodule with macrophage aggregation. pleural effusion was demonstrated on chest CT scans performed in May. As the possibility of malignant tumor could not be ruled out, an operation was undertaken. The nodular lesion showed marked coagulation necrosis, and dead parasites were noted in the vascular lumen. The parasites had the characteristic morphological features of dirofilaria immitis. Immunological studies produced a positive test result for the anti-dirofilaria immitis antibody; hence a diagnosis of pulmonary dirofilariasis was made.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/21. A case of multiple leiomyomatous lesions of the lung: an analysis of flow cytometry and hormone receptors.A 36 year old woman was admitted to our department because of a chest X-ray which showed multiple developing shadows. She underwent bilateral exploratory thoracotomies and a total 5 tumors were resected and pathologically diagnosed as benign metastasizing leiomyoma, the largest of which was positive for the progesterone receptor and negative for the estrogen receptor. A histogram of this tumor using a flow cytometer showed a diploid pattern and 4.6 percent of the s phase which was not more than that of a leiomyoma of the uterus from another patient. Two months later, she underwent a hysterectomy and bilateral salpingo-oophorectomy for treatment of the positive progesterone receptor in the pulmonary lesions. The resected uterine myoma and normal myometrium showed positive estrogen and progesterone receptors. For the subsequent 28 months she has been free of any further symptoms. Benign metastasizing leiomyoma of the uterus is a rare disease and very interesting because of its histological benignity and hormonal dependency. However, according to the literature, it is often confused in entity due to the fact that normal lung tissue also possesses hormone receptors. Considering our data on hormone receptors, it is rational to think that multiple leiomyomatous lesions in the lung should only be diagnosed as benign metastasizing leiomyomas when they possess positive estrogen and progesterone receptors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/21. Uterine adenomyoma with lymphoid infiltration simulating lymphoma.BACKGROUND: Uterine leiomyoma with lymphoid infiltration is a rare disease that simulates malignant lymphoma with only nine cases reported to date. We describe the first case of uterine adenomyoma with lymphoid infiltration simulating lymphoma. CASE: The specimen resected from a 30-year-old Chinese woman was a well-defined firm nodule measuring 5 x 5.5 x 5.5 cm. The cut surface was similar to that of adenomyoma, which contained dark brown spots and a cyst. Microscopically, the tumor comprised smooth muscle cells intermixed with many lymphocytes. Many lymphoid follicles were present, just locating beside one side of thin-walled blood vessels and protruding into the vessels. Endometrial-type glands and stroma were visible in the tumor. CONCLUSION: We describe the first case of uterine adenomyoma with lymphoid infiltration simulating lymphoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/21. Magnetic resonance experience of a twin pregnancy with a normal fetus and hydatidiform mole: a case report.A case of twin pregnancy with a coexistent hydatidiform mole after in vitro fertilization is presented. Prenatal magnetic resonance (MR) imaging at 20 gestational weeks demonstrated a normal fetus and a distinct junction between the molar tissue and myometrium suggesting no evidence of myometrial invasion. Clinically, the rare disease entity involves a high risk of maternal complications and intrauterine fetal death. The application of ultrafast MR imaging for prenatal examination provides important additional information for prenatal counseling and obstetric management.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/21. Papillary adenofibroma of the uterus. Report of a case involved by adenocarcinoma and review of the literature.An unusual case of uterine papillary adenofibroma involved by an invasive well-differentiated adenocarcinoma is reported. The diagnosis was established using a hysterectomy specimen from a 68-year-old woman who was initially examined for abdominal pain and vaginal bleeding. The tumor was a broad-based polypoid mass composed of papillary projections into clefts and cysts. These papillations were covered by a variety of epithelial types and focally involved by an infiltrating adenocarcinoma. The stroma was fibrous and collagenized with variable numbers of benign fibroblasts. The papillary adenofibroma is considered to be a benign mixed tumor of Mullerian origin. Fifteen other cases of uterine adenofibroma have been reported in the literature and only one of these was focally involved by an adenocarcinoma. The clinical and histologic features of this rare disease are reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/21. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus.Perivascular epithelioid tumor (PEComa), a recently defined tumor, is a very rare disease affecting various organs, most often the uterus. This tumor displays a variety of histologic and clinical features and at this point is regarded as a tumor with uncertain malignant potential. A 9-year-old girl with abdominal pain and vaginal spotting was diagnosed with PEComa of the uterus with metastasis. She received chemotherapy comprising vincristine, ifosfamide, and doxorubicin, as well as radiotherapy after surgery. After this multimodal treatment, there was no evidence of recurrence or further metastasis. She remains disease-free 1.5 years after her initial diagnosis. PEComa of the uterus displaying malignant characteristics may have a more favorable response to more aggressive therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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